S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose

Zusammenfassung

Die idiopathische pulmonale Fibrose (IPF) ist eine schwerwiegende und in der Regel zum Tod führende Erkrankung, die bisher nur unzureichend behandelt werden kann. Empfehlungen zur Diagnostik und Therapie wurden erstmals im ATS-ERS-Statement im Jahr 2000 publiziert [1]. Seither haben sich die diagnostischen Standards geändert und es liegen zahlreiche Therapiestudien zu diesem Krankheitsbild vor, die es erforderlich machten, die bestehenden Empfehlungen zu überarbeiten und eine wissenschaftlich begründete Leitlinie zu erstellen. Diese wurde von einer internationalen Expertengruppe in den Jahren 2006 – 2010 erarbeitet und publiziert [2]. Die folgenden Ausführungen beinhalten eine Übersetzung wesentlicher Inhalte der Originalleitlinie sowie die Interpretation und Adaptation der Empfehlungen an die speziellen Belange des deutschen Gesundheitssystems, wobei auch neue wissenschaftliche Erkenntnisse Berücksichtigung fanden. Die Deutsche Leitlinie zur Diagnostik und Therapie der IPF beruht auf einer Initiative deutscher Experten unter der Schirmherrschaft der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) und auf den Ergebnissen einer Konsensuskonferenz, die am 3. 12. 2011 in Bochum unter Supervision der „Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF)“ abgehalten wurde. Die Mehrzahl der Empfehlungen der internationalen Leitlinie konnten dabei übernommen werden. Basierend auf aktuellen Studienergebnissen, die zum Zeitpunkt der Verabschiedung der internationalen Leitlinie noch nicht bzw. nicht vollständig vorlagen wurden die schwach negativen Empfehlungen für die Antikoagulantientherapie und die Kombinationstherapie mit Prednison, Azathioprin und N-Acteylcystein in stark negative Empfehlungen umgewandelt, während für Pirfenidon, welches inzwischen in der Europäischen Union zugelassen ist, eine schwach positive Therapieempfehlung ausgesprochen wurde.

Abstract

Idiopathic pulmonary fibrosis is a fatal lung disease with a variable and unpredictable natural history and limited treatment options. Since publication of the ATS-ERS statement on IPF in the year 2000 diagnostic standards have improved and a considerable number of randomized controlled treatment trials have been published necessitating a revision. In the years 2006 – 2010 an international panel of IPF experts produced an evidence-based guideline on diagnosis and treatment of IPF, which was published in 2011. In order to implement this evidence-based guideline into the German Health System a group of German IPF experts translated and commented the international guideline, also including new publications in the field. A consensus conference was held in Bochum on December 3^rd 2011 under the protectorate of the “Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP)” and supervised by the “Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften” (AWMF). Most recommendations of the international guideline were found to be appropriate for the german situation. Based on recent clinical studies “weak negative” treatment recommendations for pirfenidone and anticoagulation were changed into “weak positive” for pirfenidone and “strong negative” for anticoagulation. Based on negative results from the PANTHER-trial the recommendation for the combination therapy of prednisone plus azathiorpine plus N-acetlycsteine was also changed into strong negative für patients with definite IPF. This document summarizes essential parts of the international IPF guideline and the comments and recommendations of the German IPF consensus conference.

^* geteilte Autorenschaft

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